Clinical immunology: PID Pt 1

Question 1

What does SPUR stand for?

Answer 1

Serious infections (unresponsive to oral antibiotics) 
Persistent infections (Early structural damage)
Unusual infections (unusual organisms, unusual sites)
Recurrent infections

Question 2

Features that may suggest primary immune deficiency

Answer 2

Weight loss or failure to thrive
Severe skin rash
Chronic diarrhoea
Mouth ulceration
Unusual autoimmune disease
Cancer
Family history

Question 3

Classifications of immunodeficiency disorders

Answer 3

Primary and Secondary

Question 4

Features of secondary immunodeficiency disorders

Answer 4

Common
Often subtle
Often involves more than one component of immune system

Question 5

Conditions associated with secondary immune deficiency

Answer 5

Infections: HIV, measles
Treatment interventions: Immunosuppresive therapy, Corticosteroids, Anti-cancer agents
Malignancy: Cancers f the immune system, Metastatic tumours
Biochemical and nutritional disorders: Malnutrition, Type 1/2 diabetes

Question 6

Primary immunodeficiency disorders (PIDs)

Answer 6

Pulmonary complications cause significant morbidity and mortality in patients with PIDs.

Question 7

Upper respiratory system complications of PIDs

Answer 7

Sinusitis
Otitis media
Laryngeal angioedema

Question 8

Lower respiratory system compications of PIDs

Answer 8

Malignancies
ILD
Pneumonia
Bronchitis, Bronchiectasis

Question 9

What is the most frequent PID associated with sinusitis and otitis media?

Answer 9

Primary antibody deficiency (PAD):
~Selective IgA deficiency
~Common variable immunodeficiency
~Specific antibody deficiency

Question 10

Examples of Primary immunodeficiency disorders with significant respiratory tract complications:
Complement system disorders

Answer 10

Should be considered in patients with laryngeal angioedema:

- Hereditary angioedema (HAE)

Question 11

Pneumonia is one of the most frequent manifestations of what PIDs?

Answer 11

Primary antibody deficiency (PAD)
Complement system disorders
Congenital phagocytosis deficiency
Combined immunodeficiencies

Question 12

What cells are involved in the innate immune system?

Answer 12

Macrophages
Mast cells
NK cells

Question 13

What proteins are involved in the innate immune system?

Answer 13

Complement
Acute phase proteins
Cytokines

Question 14

What are PAMPs?

Answer 14

Pathogen-associated molecular patterns (PAMPs) are molecular structures or molecules that are shared by most pathogenic bacteria and some viruses.

Question 15

Function of the innate immune system

Answer 15

Rapid clearance of microorganisms
Stimulates the acquired immune response
Buys time while the acquired immune system is mobilised.

Question 16

Function of Neutrophils and Monocyte/Macrophages

Answer 16

Initiation and amplification of the inflammatory response
Scavenging of cellular and infectious debris
Ingest and kill microorganisms
Produce inflammatory molecules which regulate other components of the immune system
Resolution and repair

Question 17

Where can defects occur in neutrophil development?

Answer 17

When the monocyte differentiates into the neutrophil

Question 18

What is Severe congenital neutropenia?

Answer 18

rare genetic disorder

Type 1 is the most common form and is inherited in an autosomal dom. manner

Question 19

What would you expect if a patient’s phagocytes were unable to bind to the endothelial adhesion molecules?

Answer 19

Failure to recognise activation makers expressed on endothelial cells.
Neutrophils are mobilised, but cannot exit bloodstream.

Question 20

What are the clinical features if a patient’s phagocytes were unable to bind to the endothelial adhesion molecules?

Answer 20

Recurrent bacterial and fungal infections
Very high neutrophil count in the blood
Infection found in deep tissue, NB: no pus formaiton.

Question 21

What is leukocyte adhesion deficiency?

Answer 21

Rare autosomal recessive primary immunodeficiency
Results in failure of neutrophil adhesion and migration
Clinical picture is characterised by marked leukocytes and localised bacterial infections

Question 22

What is chronic granulomatous disease (CGD)

Answer 22

Deficiency of the intracellular killing mechanism of phagocytes - absent respiratory burst.
Results in the inability to generate oxygen and impaired killing of intracellular micro-organisms.

Question 23

Clinical features of CGD

Answer 23

Recurrent deep bacterial infections
Failure to thrive
Granuloma formation

Question 24

What defects in macrophage killing are caused by the Mycobacteria species?

Answer 24

Mycobacteria species are clever by hiding within immune cells